Solans R, Bosch J A, Pérez-Bocanegra C, Selva A, Huguet P, Alijotas J, Orriols R, Armadans L, Vilardell M
Department of Internal Medicine, Department of Pathology, Department of Pneumology and. Department of Preventive Medicine, Vall d'Hebrón University General Hospital, 08035 Barcelona, Spain.
Rheumatology (Oxford). 2001 Jul;40(7):763-71. doi: 10.1093/rheumatology/40.7.763.
To study the clinical spectrum and evolution of Churg-Strauss syndrome in order to assess the clinicopathological features of the disease, the response to treatment and the long-term outcome.
Thirty-two patients with proven allergic and granulomatous angiitis (Churg-Strauss syndrome) and followed up at a single institution were evaluated. They were recruited between 1977 and 1999 from internal medicine departments. Data were obtained retrospectively from medical files in 15 cases and prospectively, using a standardized form, for the remaining patients.
All patients had asthma and hypereosinophilia. The lungs, skin and peripheral nervous system were the organs most frequently involved. Antineutrophil cytoplasmic antibodies with antimyeloperoxidase specificity (MPO-ANCA) were detected in 77.8% of tested patients but they were not useful for monitoring disease activity. Extravascular granulomas were rarely seen in tissue biopsies. Forty per cent of the patients were treated with steroids alone. Immunosuppressive agents were added to the treatment when severe neurological, cardiac or gastrointestinal involvement was present. The outcome and long-term survival were good. Clinical relapse was rare after the first year of therapy. Dysaesthesiae of the distal limbs, neurophatic pain and cardiac failure were the most frequent sequelae.
Churg-Strauss syndrome is a rare disorder characterized by hypereosinophilia and systemic vasculitis occurring in patients with asthma and allergic rhinitis. Vasculitis commonly affects the lungs, skin and peripheral nervous system. Outcome and long-term survival is usually good with steroids alone or in combination with immunosuppressive agents. The syndrome has a low mortality rate compared with other systemic vasculitides.
研究变应性肉芽肿性血管炎（Churg-Strauss综合征）的临床谱及病情演变，以评估该疾病的临床病理特征、治疗反应及长期预后。
对32例经证实的变应性肉芽肿性血管炎（Churg-Strauss综合征）患者进行评估，这些患者均在同一机构接受随访。他们于1977年至1999年间从内科招募。15例患者的数据通过回顾性查阅病历获得，其余患者则使用标准化表格进行前瞻性数据收集。
所有患者均有哮喘和嗜酸性粒细胞增多。肺、皮肤和周围神经系统是最常受累的器官。77.8%的受试患者检测到抗髓过氧化物酶特异性抗中性粒细胞胞浆抗体（MPO-ANCA），但它们对监测疾病活动并无帮助。组织活检中很少见到血管外肉芽肿。40%的患者仅接受类固醇治疗。当出现严重的神经、心脏或胃肠道受累时，在治疗中加用免疫抑制剂。预后和长期生存率良好。治疗第一年之后临床复发很少见。四肢远端感觉异常、神经性疼痛和心力衰竭是最常见的后遗症。
Churg-Strauss综合征是一种罕见疾病，其特征为哮喘和变应性鼻炎患者出现嗜酸性粒细胞增多和系统性血管炎。血管炎通常累及肺、皮肤和周围神经系统。单独使用类固醇或与免疫抑制剂联合使用，通常预后良好且长期生存率高。与其他系统性血管炎相比，该综合征死亡率较低。
